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2008 ICD-9-CM Volume 1 Diagnosis Codes Home > Diseases Of The Nervous System And Sense Organs 320-389 > Disorders Of The Peripheral Nervous System 350-359 > Muscular Dystrophies And Other Myopathies 359.* > 2008 ICD-9-CM Diagnosis 359.1 Hereditary progressive muscular dystrophy  On October 1, 2008 the 2009 ICD-9-CM codes came into effect. Therefore, this code may be out of date. The 2009 version of ICD-9-CM 359.1 can be accessed here. A heterogeneous group of genetic disorders characterized by progressive MUSCULAR ATROPHY and MUSCLE WEAKNESS beginning in the hands, the legs, or the feet. Most are adult-onset autosomal dominant forms. Others are autosomal recessive. A heterogeneous group of inherited MYOPATHIES, characterized by wasting and weakness of the SKELETAL MUSCLE. They are categorized by the sites of MUSCLE WEAKNESS; AGE OF ONSET; and INHERITANCE PATTERNS. A heterogenous group of inherited muscular dystrophy that can be autosomal dominant or autosomal recessive. There are many forms (called LGMDs) involving genes encoding muscle membrane proteins such as the sarcoglycan (SARCOGLYCANS) complex that interacts with DYSTROPHIN. The disease is characterized by progressing wasting and weakness of the proximal muscles of arms and legs around the HIPS and SHOULDERS (the pelvic and shoulder girdles). A progressive disorder involving pelvic and scapular girdle muscles marked by weakness of proximal muscles of hip and shoulder areas with variable psychiatric disorders and occasional mild mental retardation. Type IA (LGMD1A) Synonym: proximal limb-girdle muscular dystrophy type 1A A slowly progressive form of muscular dystrophy affecting the proximal limb muscles but sparing the face. (159000) Type IB (LGMD1B) Synonym: proximal limb-girdle muscular dystrophy type 1B A slowly progressive form of muscular dystrophy differing from LGMD1A by the nasal quality of speech. (OMIM 159001) Type 2 (LGMD2, LGMD2A) Synonyms: Leyden-Mobius syndrome distal muscular dystrophy pelvofemoral muscular dystrophy pelvofemoral syndrome tibial muscular dystrophy Muscular dystrophy involving first the pelvic or, less commonly, shoulder girdle with occasional pseudohypertrophy of the calves with onset usually in childhood but sometimes in later stages of life. Muscle wasting tends to be asymmetric when the upper limbs are first involved. Progression of disease varies with inability to walk usually taking place at about 20 to 30 years of onset. Facial weakness and contractures may occur later. (OMIM 253600) Type 3 (LGMD3, LGMD2B) A slowly progressive form of muscular dystrophy involving the pelvic girdle with onset in the late teens. (OMIM 253601) An autosomal dominant degenerative muscle disease characterized by slowly progressive weakness of the muscles of the face, upper-arm, and shoulder girdle. The onset of symptoms usually occurs in the first or second decade of life. Affected individuals usually present with impairment of upper extremity elevation. This tends to be followed by facial weakness, primarily involving the orbicularis oris and orbicularis oculi muscles. (Neuromuscul Disord 1997;7(1):55-62; Adams et al., Principles of Neurology, 6th ed, p1420) An autosomal dominant hereditary disease that presents in late in life and is characterized by DYSPHAGIA and progressive ptosis of the eyelids. Mutations in the gene for POLY(A)-BINDING PROTEIN II have been associated with oculopharyngeal muscular dystrophy. An X-linked recessive muscle disease caused by an inability to synthesize DYSTROPHIN, which is involved with maintaining the integrity of the sarcolemma. Muscle fibers undergo a process that features degeneration and regeneration. Clinical manifestations include proximal weakness in the first few years of life, pseudohypertrophy, cardiomyopathy (see MYOCARDIAL DISEASES), and an increased incidence of impaired mentation. Becker muscular dystrophy is a closely related condition featuring a later onset of disease (usually adolescence) and a slowly progressive course. (Adams et al., Principles of Neurology, 6th ed, p1415) Pelvic muscle weakness and atrophy associated with pseudohypertrophy of the calves. Two basic types are recognized: Duchenne muscular dystrophy (Type I) Synonym: childhood pseudohypertrophic muscular dystrophy with the onset of symptoms between the ages of 3 and 5 years, the patients becoming bedridden by age of 12 and death by age 20 to 25 years. Becker muscular dystrophy (Type II) Synonyms: adult pseudohypertrophic muscular dystrophy mild or benign X-linked recessive muscular dystrophy with onset of symptoms between the ages of 20 and 30 years and relatively normal life expectancy in most cases. Atrophic changes begin in the muscles of the pectoral girdle and trunk and extend to the extremities. As their size increases, the muscles become firm and resilient, but their strength diminishes and, eventually, atrophy ensues. Inability to rise to an upright position without turning to the side, waddling gait, and progressive weakness are the characteristic features. Pseudohypertrophy of the calf muscles is due to fibrous tissue and fat infiltration. Involvement of the heart consists of usually terminal cardiomyopathy. Fasciculation and atrophy of the tongue with dysphagia, dysarthria, and thickened nasal speech are usually associated. Usually mild and static mental retardation occurs in about 30% DMD patients most children having normal, even superior intelligence. 359.1 is a specific code that can be used to specify a diagnosis 359.1 contains 56 index entries View the ICD-9-CM Volume 1 359.* hierarchy 359.1 also known as: Muscular dystrophy: NOS distal Duchenne Erb's fascioscapulohumeral Gower's Landouzy-Déjérine limb-girdle ocular oculopharyngeal Index entries containing 359.1: Atrophy, atrophic facioscapulohumeral (Landouzy-Déjérine) 359.1 Landouzy-Déjérine 359.1 muscle, muscular 728.2 pseudohypertrophic 359.1 pseudohypertrophic 359.1 Disease, diseased - see also Syndrome Duchenne's 094.0 muscular dystrophy 359.1 pseudohypertrophy, muscles 359.1 Duchenne-Griesinger 359.1 Erb (-Landouzy) 359.1 Disorder - see also Disease neuromuscular NEC 358.9 hereditary NEC 359.1 Duchenne's disease 094.0 muscular dystrophy 359.1 pseudohypertrophy, muscles 359.1 Duchenne-Griesinger disease 359.1 Dystrophy, dystrophia 783.9 Duchenne's 359.1 Erb's 359.1 Gowers' muscular 359.1 hereditary, progressive muscular 359.1 Landouzy-Déjérine 359.1 Leyden-Möbius 359.1 muscular 359.1 congenital (hereditary) 359.0 myotonic 359.22 distal 359.1 Duchenne's 359.1 Erb's 359.1 fascioscapulohumeral 359.1 Gowers' 359.1 hereditary (progressive) 359.1 Landouzy-Déjérine 359.1 limb-girdle 359.1 myotonic 359.21 progressive (hereditary) 359.1 Charcôt-Marie-Tooth 356.1 pseudohypertrophic (infantile) 359.1 ocular 359.1 oculopharyngeal 359.1 pelvicrural atrophic 359.1 progressive ophthalmoplegic 359.1 scapuloperoneal 359.1 Erb's disease 359.1 pseudohypertrophic muscular dystrophy 359.1 Facioscapulohumeral myopathy 359.1 Gowers' muscular dystrophy 359.1 Hypertrophy, hypertrophic pseudomuscular 359.1 Landouzy-Déjérine dystrophy (fascioscapulohumeral atrophy) 359.1 Leyden-Möbius dystrophy 359.1 Myopathy 359.9 distal 359.1 facioscapulohumeral 359.1 limb-girdle 359.1 ocular 359.1 oculopharyngeal 359.1 scapulohumeral 359.1 Ocular muscle - see also condition myopathy 359.1 Paralysis, paralytic (complete) (incomplete) 344.9 muscle (flaccid) 359.9 pseudohypertrophic 359.1 pseudohypertrophic 359.1 muscle 359.1 Paresis (see also Paralysis) 344.9 pseudohypertrophic 359.1 Pseudohypertrophic muscular dystrophy (Erb's) 359.1 Pseudohypertrophy, muscle 359.1 Scapulohumeral myopathy 359.1 Syndrome - see also Disease scapuloperoneal 359.1